SUMMARY Neutropenia is a generic term that indicates a reduction of neutrophils below the threshold for age and race. This condition encompasses a number of diseases with a wide range of duration and severity. In the present paper, the approach to diagnosis and treatment of neutropenia has been reviewed and implemented with the knowledge acquired during the last decade, by a group of experts, 10 years after the first publication. The diagnostic itinerary highlights the most important tools available to define the type of neutropenia and updates the list of genes causative of the disease. In addition, the present paper underlines the progresses towards a better definition of “primary autoimmune neutropenia” without remission which often hides different diseases. Moreover, indications on how to speed up neutropenia diagnosis and the indications to perform the bone marrow examination in the genetic forms, are given. The management and treatment of the “well-known” diseases and “special situations” are also reviewed giving literature derived and expert opinion-based suggestions tailored on the single patient/diagnosis.